Wilms tumor, also known as nephroblastoma, is a type of cancer that initially appears in the kidneys and is the most common type of kidney cancer in children, accounting for approximately 90% of cases.
It may also present in adults, although this is much less common. The typical Wilms tumor patient is approximately 3 years old, and it is rare for a patient older than 8 years old to be newly diagnosed with Wilms tumor.
Most cases of Wilms tumor are unilateral and affect just one of the two kidneys, usually with a single localized tumor. Occasionally, approximately 1 in 15 children with this type of cancer will have multiple tumors in the single kidney. Additionally, about 5% of cases have bilateral disease, in which both kidneys are affected by tumors.
In early stages of the disease symptoms are not usually problematic and, as a result, Wilms tumors often grow to be quite large before they are detected. However, in most cases diagnosis is made before the tumor metastasizes and other organs are not usually affected.
Symptoms that may be indicative of Wilms Tumor are:
- Abdominal pain and swelling
- Loss of appetite
- Urine discoloration
There may also be a growth localized to one side of the body in the abdominal region, due to the tumor growth. This may be detectable upon physical examination.
Initially, Wilms tumor may be suspected when a child presents with symptoms characteristic of the disease. The physician will then ask about medical history and family history of cancer, and conduct a physical exam to find the indicative presence of abdominal mass or hypertension.
Several scans and imaging tests may be requested to determine the cause of the symptoms and if Wilms tumor is to blame. These tests may include:
- Blood test for signs of anemia and levels of urea and nitrogen
- Computer Tomography (CT) scan of abdominal region
- Creatinine clearance
- Ultrasound of abdominal region
- X-Ray of abdominal region and chest
Diagnosis can only be confirmed with a biopsy of tumor cells, which are examined microscopically in a laboratory environment. If evidence of Wilms tumor is discovered, further tests may be required to confirm if the disease has metastasized to other areas of the body.
There are two main types of Wilms tumor, according to their microscopic appearance:
- Favorable histology refers to cells that don’t display normal qualities but show no evidence of anaplasia. This type accounts for the majority of children with Wilms tumor and has a good prognosis.
- Unfavorable histology refers to cells with wide variability, characterized by large and distorted DNA in the cell nucleus, known as anaplasia. This is a less common type and is associated with a poorer prognosis.
The prognosis of Wilms tumor is usually quite positive, particularly when the disease is discovered early, before it has metastasized to other areas of the body. Localized tumors are associated with a 90% cure rate with 8 out of 10 patients surviving long term.
The primary tumor is usually removed as early as practicable, to prevent the spread to other organs such as the lungs, liver, bones and brain. This may affect the renal function of the kidneys, but the benefits are generally considered to greatly outweigh the risks.
Survivors or Wilms tumor are also at an increased risk of second tumors. This may include bone and soft-tissue sarcomas, lymphoma, melanoma, leukemia and cancer of the breast and gastrointestinal tract.